Congenital QT Syndrome Research Articles

Ventricular Tachycardia.

This scenario was developed to educate emergency medicine residents on the diagnosis and management of ventricular tachycardia (VT) that is refractory to single dose anti-arrhythmic management. Electrical storm, defined as three or more episodes of sustained VT, ventricular fibrillation, or appropriate shocks from an implantable cardioverter defibrillator within 24 hours,1 has a mortality rate up to 14% in the first 48 hours.2 Ventricular tachycardia may present in a heterogenous fashion, not only with stable versus unstable clinical presentations, but also with different electrocardiographic morphologies and etiologies.1 Understanding how to rapidly diagnose, treat, and utilize second or third-line treatments is vital in the setting of refractory ventricular tachycardia rather than relying on the success of first-line agents. Appreciation for what medications are readily available in your crash cart and medication dispensing cabinet is critical for timely management for refractory ventricular tachycardia. At the conclusion of the simulation session, learners will be able to: 1) identify the different etiologies of VT, including structural heart disease, acute ischemia, and acquired or congenital QT syndrome; 2) describe confounding factors of VT, such as electrolyte abnormalities and sympathetic surge; 3) describe how to troubleshoot an unsuccessful synchronized cardioversion, including checking equipment connections, increasing delivered energy, and changing pad placement; 4) compare and contrast treatments of VT based on suspected underlying etiology; 5) describe reasons to activate the cardiac catheterization lab other than occlusive myocardial infarction; and 6) identify appropriate disposition of the patient to the cardiac catheterization lab. This session was conducted using high-fidelity simulation, followed by a debriefing session and lecture on the diagnosis, differential diagnosis, and management of VT. Debriefing methods may be left to the discretion of participants, but the authors have utilized advocacy-inquiry techniques. This scenario may also be run as an oral board case. Our residents are provided a survey at the completion of the debriefing session so they may rate different aspects of the simulation, as well as provide qualitative feedback on the scenario. The local institution's simulation center's electronic feedback form is based on the Center of Medical Simulation's Debriefing Assessment for Simulation in Healthcare (DASH) Student Version Short Form3 with the inclusion of required qualitative feedback if an element was scored less than a 6 or 7. Twelve learners completed a feedback form. This session received 6 and 7 scores (consistently effective/very good and extremely effective/outstanding, respectively) other than three isolated 5 scores. The lowest average score was 6.67 for "Before the simulation, the instructor set the stage for an engaging learning experience." The highest average score was 7 for "The instructor helped me see how to improve or how to sustain good performance." The form also includes an area for general feedback about the case at the end. Illustrative examples of feedback include: "Excellent care and debrief." Specific scores are available upon request. This is a cost-effective method for reviewing VT diagnosis and management. The case may be modified for appropriate audiences, such as describing what medications may be readily available in a free-standing emergency department or pre-hospital setting. Medical simulation, ventricular tachycardia, cardiac emergencies, dysrhythmias, cardiology, emergency medicine.

Congenital QT Syndrome Causing Syncope in Children, A Case to be proposed

Congenital QT Syndrome Causing Syncope in Children, A Case to be proposed

Fatal arrhythmia mistaken as anxiety and pseudo-seizures in an adolescent

Introduction Congenital Long QT syndrome (LQTS) is a disorder of ventricular myocardial repolarization with potential to cause symptomatic ventricular arrhythmias and sudden cardiac death (SCD). This case presents a young female presenting in cardiac arrest due to underlying LQTS. Patient had symptoms that was misdiagnosed as anxiety and pseudo-seizures. Case Report A 20-year-old female, history of pseudo-seizures, depression and anxiety, was air lifted for cardiac arrest. She was found down outside a health clinic; unwitnessed loss of consciousness, no pulse or respirations requiring CPR and 2 defibrillations. Reportedly was in Ventricular Fibrillation and required intubation by EMS for GCS of 3 and desaturations …

Case report of congenital long QT syndrome with congenital complete atrioventricular block and malignant ventricular arrhythmia

Case report of congenital long QT syndrome with congenital complete atrioventricular block and malignant ventricular arrhythmia

Spontaneous Variability of QTc Interval in Patients with Congenital Long QT Syndrome

Introduccion: La medicion del intervalo QTc es una herramienta indispensable para diagnosticar y estratificar el riesgo de eventos cardiacos adversos en los pacientes (p) con los sindromes de QT largo (SQTL) hereditarios. Los intervalos QTc pueden variar en forma ostensible y prolongarse de manera intermitente solo antes, durante o despues de un evento arritmico. Objetivos: Determinar si, en los pacientes con SQTL, existen variaciones significativas de los intervalos QTc cuando se los mide en varias oportunidades. Material y metodos: Se midieron la duracion de los intervalos QTc en tres ECG registrados cada 7 dias, en 25 pacientes con SQTL y 15 controles sanos. Resultados: Los intervalos QTc variaron de manera significativa en ambos grupos. Los valores minimos y maximos de QTc fueron de 492 mseg (rango intercuartil [RIC] 25-75: 463,5-501,5) y 522 mseg (RIC: 503,5-543) en pacientes con SQTL (p < 0,0001), respectivamente. En los individuos sanos, dichos valores fueron de 409 mseg(RIC: 395-418) y 423 mseg (RIC: 413431) (p < 0,001). En el 56% de los pacientes, las mediciones de los intervalos QTc oscilaron por encima y por debajo de 500 mseg. El 24% tuvo un intervalo QTc siempre por debajo de 500 mseg y el 20% siempre por encima. El intervalo QTc siempre fue mayor de 500 mseg en el 50% de los sintomaticos y en el 10,5% de los asintomaticos. Conclusiones: En los pacientes con SQTL, los intervalos QTc varian significativamente. Una unica determinacion no es suficiente para estratificar el riesgo.

Is LQTS OK During Pregnancy

Patients with congenital long QT syndrome (LQTS) exhibit prolongation of the QT interval on electrocardiograms (see Table 1) and are at risk for sudden

Multi-undulant T-U-wave, sinus bradycardia and long QT syndrome: a possible phenotype of mutant genes controlling the inward potassium rectifiers.

Inward rectifying potassium currents (Ikr and Iks) during phase 3 repolarization of the myocyte from the beginning to the end of repolarization of the myocardial syncytium will inscribe a T-U-wave on the surface electrocardiogram (ECG). Type two congenital long QT syndrome (LQT2) is a phenotype of human ether-a-go-go-related gene (HERG) mutation on the chromosome 7q 35-36. Type one congenital long QT syndrome (LQT1) is a phenotype of KvLQT1 mutation on the chromosome 11p15.5. Both LQT1 and LQT2 relate with inward rectifying potassium currents and is repolarization related, therefore, it is speculate that patients of LQT1 and LQT2 may have an abnormal T-U-wave on their surface ECG. To two probands of congenital LQT, 8 patients of structural heart disease treated by open heart surgery, 13 patients of structural heart disease without open-heart surgery, and 10 patients of normal controls, 24 hour-Holter monitoring was performed from July to December 1996. Their corrected QT interval (QTc) as well as the RR interval of every heart beat was calculated by a computer. The results showed that all 33 patients exhibited beat-by-beat fluctuation of their QTc and RR daily. The RR intervals of these two probands of congenital LQT were somewhile more than 1200 ms during circadian waking time, while 31 cases without LQT showed their RR prolongation only during the circadian sleeping time. A multi-undulant T-U-wave, or a beat-to-beat changing of vectors or amplitudes of their T-U-wave observed in these two probands of congenital LQT, were not observable in those 31 patients without congenital LQT. Therefore, we concluded that multi-undulant T-U-wave, sinus bradycardia and a longer QTc was a phenotype of the mutated genes which control the inward rectifying potassium currents during phase 3 repolarization.